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The Value of Individual Microscopic Features in Diagnosis of Cutaneous Lupus Erythematosus (LE)

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The Value of Individual Microscopic Features in Diagnosis of Cutaneous Lupus Erythematosus (LE) International Journal of Pathology; 2005; 3(2): 76-80 The Value of Individual Microscopic Features in Diagnosis of Cutaneous Lupus Erythematosus (LE) Ambreen Moatasim and Anwar Ul Haque Department of Pathology, Pakistan Institute of Medical Sciences Objective: The objective of this study was to determine the frequency-based value of various histologic features encountered in skin biopsies evaluated for LE. Study Design and Setting: This descriptive study was performed over a period of one year at Pathology Department of Pakistan Institute of Medical Sciences, Islamabad. Subjects: The total number of cases was 50, selected through convenience non-probability sampling. Methods: A histopathologist and several dermatologists evaluated each case of skin biopsy together on multi-head microscope and TV monitors and combined diagnoses were made. Later on all slides were reviewed by a senior pathologist. A criterion was fixed for examining each slide. 12 histologic parameters were studied in each. Each of these parameters was further graded into nil, mild and prominent. All the data was entered into SPSS and frequencies calculated. Results: Hyperkeratosis, basal cell damage and collagen damage were present in all 50 (100%) cases while epidermal atrophy in 49 (98%) and periappendigeal and perivascular inflammation were seen in 48 (96%) cases each. Epidermal atrophy was prominent in 90 % cases, while basal cell damage, collagen damage, periappendigeal inflammation and hyperkeratosis were prominent in 78%, 62%, 56% and 54% cases respectively. Basement membrane deposit was prominent in 14%, while perivascular inflammation and keratin plugging were both prominent in 40% cases each. Conclusion: Certain histological features are distinctive enough to render a reasonably accurate diagnosis of Cutaneous LE in most of the cases, and include epidermal atrophy, basal cell damage, collagen damage, periappendigeal inflammation and hyperkeratosis. Key words: Lupus erythematosus, Cutaneous, Diagnosis, Microscopic features. Introduction Materials and Methods Lupus erythematosus (LE) is a multisystem, This descriptive study was performed at autoimmune disease characterized by the formation of Pathology Department of Pakistan Institute of Medical autoantibodies against various cellular constituents. Sciences. Duration of study was one year. The total Skin is one of the target organs invariably affected by number of cases was 50, selected through convenience the disease1 and cutaneous lesions make up 3 out of non-probability sampling. Patients were selected 11 criteria set by American College of Rheumatology regardless of age, sex or site of the lesion. All cases for diagnosing systemic lupus erythematosus; these with a clinical suspicion of cutaneous LE, in whom being malar rash, discoid lesions and photosensitivity2. skin biopsy was performed to confirm the diagnosis, Cutaneous lupus erythematosus refers to were included in this study. Also only those cases cutaneous lesions of LE, whether occurring exclusively were selected in whom DIF or serological tests were or as part of a systemic disease.3 The diagnosis of not performed. Poorly processed slides with a lot of cutaneous LE requires clinicohistological data along artifacts were excluded from the study. All skin with direct immunofluoresecence (DIF) and biopsies with a high index of suspicion of cutaneous serological reactions. In Pakistan, however, due to LE were routinely processed and stained with restricted resources it is not possible to perform Hematoxylin and Eosin. The diagnosis was rendered serology and DIF in every case. in weekly Dermatopathology session held every The aim of this study was to find out the value Friday together with several dermatologists on multi- of individual histologic features that can help in head microscope attached with TV monitors. diagnosing cutaneous LE in the absence of DIF or Thorough histopathological and clinical correlation serology and hence facilitate patient care by making was carried out. Later all the slides were reviewed by a accurate diagnosis without resorting to expensive senior pathologist. A criterion was set for examining diagnostic modalities. each slide. 12 parameters were studied in each case 76 International Journal of Pathology; 2005; 3(2): 76-80 including Hyperkeratosis, Parakeratosis, Keratin respectively. Basement membrane deposit was plugging, Epidermal atrophy, Acanthosis, Basal cell prominent in 14%, while both perivascular damage, Basement membrane deposit, Pigmentory inflammation and keratin plugging were seen incontinence, Extravasation of RBCs, Periappendigeal prominently in 40% cases each (Table 1) and Perivascular inflammation & Collagen damage. Each of these parameters was further graded into nil, mild and prominent depending upon the certainty Collagen damage 50 with which a particular feature was present. All the Perivascular inflammation 48 data was entered into SPSS and frequencies calculated. Results were presented in the form of tables and Periappendigeal inflammation 48 graphs. Extravasation of RBCs 26 Pigmentory incontinence 34 Results Basement membrane deposit 41 Hyperkeratosis, basal cell damage and Basal cell damage 50 collagen damage were present in all 50 (100%) cases Acanthosis 15 while epidermal atrophy was seen in 49 (98%) and Epidermal atrophy 49 periappendigeal and perivascular inflammation were Keratin plugging 44 seen in 48 (96%) cases each. The least frequent Parakeratosis 8 parameter was parakeratosis (16%) followed by acanthosis (30%) (Figure 1). Hyperkeratosis 50 The epidermal atrophy was prominent in 90 % 0204060 cases, while basal cell damage, collagen damage, periappendigeal inflammation and hyperkeratosis Fig. 1: Frequencies of Individual Microscopic were prominent in 78%, 62%, 56% and 54% cases Features Table: 1: Gradation of Individual Microscopic Features into Nil, Mild and Prominent Nil Mild Prominent Total Hyperkeratosis 0 23 (46%) 27 (54%) 50 (100%) Parakeratosis 42 (84%) 3 (6%) 5 (10%) 50 (100%) Keratin plugging 6 (12%) 24 (48%) 20 (40%) 50 (100%) Epidermal atrophy 1 (2%) 4 (8%) 45 (90%) 50 (100%) Acanthosis 35 (70%) 11 (22%) 4 (8%) 50 (100%) Basal cell damage 0 11 (22%) 39 (78%) 50 (100%) Basement membrane deposit 9 (18%) 34 (68%) 7 (14%) 50 (100%) Pigmentory incontinence 16 (32%) 22 (44%) 12 (24%) 50 (100%) Extravasation of RBCs 24 (48%) 20 (40%) 6 (12%) 50 (100%) Periappendigeal inflammation 2 (4%) 20 (40%) 28 (56%) 50 (100%) Perivascular inflammation 2 (4%) 28 (56%) 20 (40%) 50 (100%) Collagen damage 0 19 (38%) 31 (62%) 50 (100%) 77 International Journal of Pathology; 2005; 3(2): 76-80 that displays systemic, cutaneous or both systemic and cutaneous manifestations. Skin is one of the target organs invariably affected by the disease1 and cutaneous lesions make up 3 out of 11 criteria set by American College of Rheumatology for diagnosing systemic Fig. 2: Epidermal Atrophy and lupus erythematosus; Fig. 5: Basement Membrane Keratin Plugging these being malar rash, Deposit discoid lesions and and Pigmentory Incontinence photosensitivity2. Cutaneous lupus erythematosus (LE) refers to cutaneous lesions of LE, whether occurring exclusively or as part of a systemic disease.3 Over time several classifications have been proposed for cutaneous lupus erythematosus3-6. The classification proposed Fig. 3: Epidermal Atrophy and by Sontheimer et al.,3 based on clinical Hyperkeratosis Fig. 6: Ropy Collagen morphology and specific histologic findings of the disease is more accepted and divides Cutaneous LE into 3 main forms that is, Chronic Cutaneous LE (CCLE), Subacute Cutaneous LE (SCCLE) and Acute Cutaneous LE (ACLE). Gilliam however expanded this classification on the basis of Specific and Non- Specific clinical and histological features found in LE patients.7, 8 For every patient with cutaneous lesions of LE, an initial workup is mandatory to exclude the possibility of a systemic disease and should include a battery of blood tests, serologic studies, histology of the cutaneous lesions and direct immunofluoresecence. As far as the serologic tests are concerned, the titres of Anti-nuclear antibody (ANA), anti-Ro (SSA)/La (SSB), and Anti-cardiolipin Fig. 4: Basal Cell Vacuolization antibody (ACL) are usually raised. Also there is false positive VDRL test for syphilis. Histologically, Acute Cutaneous LE (ACLE) in early Discussion stages shows non- specific features including papillary dermal Lupus erythematosus (LE) is an edema and perivascular lymphocytic infiltrate. However, in an autoimmune connective tissue disorder established case of ACLE, one usually finds epidermal atrophy, 78 International Journal of Pathology; 2005; 3(2): 76-80 liquefactive degeneration, and papillary shows similar histological features differing only in that there is dermal fibrinoid deposition. The most prominent epidermal atrophy, minimal or non-existent follicle common form of Chronic Cutaneous LE plugging or thickening of the basal membrane, and mild to moderate (CCLE) is mononuclear cell infiltrate, restricted to the superficial dermis in Discoid LE (DLE) that histologically SCLE lesions. displays following features: Direct immunofluoresecence (DIF) is not absolute for 1) hyperkeratosis with follicular plugging; diagnosis of LE but is helpful when other diagnostic features are 2) thinning and flattening of the stratum absent. DIF detects IgG, IgM and complement deposition at Malpighi's, 3) hydropic degeneration of dermoepidermal junction. basal cells; The current study focuses on diagnosis of Cutaneous LE histologically
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